Pck2 phosphoenolpyruvate carboxykinase 2 (mitochondrial) [Mus musculus (house mouse)] - Gene

Summary

Official Symbol: Pck2provided by MGI
Official Full Name: phosphoenolpyruvate carboxykinase 2 (mitochondrial)provided by MGI
Primary source: MGI:MGI:1860456
See related: Ensembl:ENSMUSG00000040618 AllianceGenome:MGI:1860456
Gene type: protein coding
RefSeq status: VALIDATED
Organism: Mus musculus
Lineage: Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Mus; Mus
Also known as: PEPCK-M; 1810010O14Rik; 9130022B02Rik
Summary: Enables phosphoenolpyruvate carboxykinase (GTP) activity. Involved in gluconeogenesis. Located in mitochondrion. Is active in mitochondrial matrix. Is expressed in several structures, including alimentary system; central nervous system; genitourinary system; heart; and sensory organ. Orthologous to human PCK2 (phosphoenolpyruvate carboxykinase 2, mitochondrial). [provided by Alliance of Genome Resources, Nov 2024]
Expression: Ubiquitous expression in ovary adult (RPKM 31.2), spleen adult (RPKM 15.0) and 26 other tissues See more
Orthologs: human all
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Genomic context

Location:: 14 C3; 14 28.19 cM

Exon count:: 10

Annotation release	Status	Assembly	Chr	Location
RS_2024_02	current	GRCm39 (GCF_000001635.27)	14	NC_000080.7 (55777721..55787477)
108.20200622	previous assembly	GRCm38.p6 (GCF_000001635.26)	14	NC_000080.6 (55540263..55550020)

Chromosome 14 - NC_000080.7 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Go to reference sequence details

Genomic Sequence:

Go to nucleotide: Graphics FASTA GenBank

Expression

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See details

Project title: Mouse ENCODE transcriptome data Mouse ENCODE transcriptome data
Description: RNA profiling data sets generated by the Mouse ENCODE project.
BioProject: PRJNA66167
Publication: PMID 25409824
Analysis date: n/a

Bibliography

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GeneRIFs: Gene References Into Functions

What's a GeneRIF?

PCK2 maintains intestinal homeostasis and prevents colitis by protecting antibody-secreting cells from oxidative stress.
Title: PCK2 maintains intestinal homeostasis and prevents colitis by protecting antibody-secreting cells from oxidative stress.
DNA 5mC and RNA m[6]A Collaborate to Upregulate Phosphoenolpyruvate Carboxykinase 2 for Kupffer Cell Activation.
Title: DNA 5mC and RNA m(6)A Collaborate to Upregulate Phosphoenolpyruvate Carboxykinase 2 for Kupffer Cell Activation.
Role of PCK2 in the proliferation of vascular smooth muscle cells in neointimal hyperplasia.
Title: Role of PCK2 in the proliferation of vascular smooth muscle cells in neointimal hyperplasia.
Lin28a promotes pathological cardiac hypertrophy and glycolytic reprograming, at least in part, by binding to and stabilizing Pck2 mRNA.
Title: Lin28a Regulates Pathological Cardiac Hypertrophic Growth Through Pck2-Mediated Enhancement of Anabolic Synthesis.
Downregulation of PCK2 remodels tricarboxylic acid cycle in tumor-repopulating cells of melanoma
Title: Downregulation of PCK2 remodels tricarboxylic acid cycle in tumor-repopulating cells of melanoma.
Lactate maintains/induces populations of postnatal neuronal progenitors/neural stem cells in a PEPCK-M-dependent manner
Title: Neuronal Progenitor Maintenance Requires Lactate Metabolism and PEPCK-M-Directed Cataplerosis.
Amino acid limitation and ER stress inducers, conditions that activate the amino acid response (AAR) and the unfolded protein response (UPR), stimulate PCK2 gene transcription in tumor cell lines.
Title: Mitochondrial phosphoenolpyruvate carboxykinase (PEPCK-M) is a pro-survival, endoplasmic reticulum (ER) stress response gene involved in tumor cell adaptation to nutrient availability.
PEPCK-M expression partially rescued defects in lipid metabolism, gluconeogenesis and TCA cycle function impaired by PEPCK-C deletion, while approximately 10% re-expression of PEPCK-C normalized most parameters.
Title: PEPCK-M expression in mouse liver potentiates, not replaces, PEPCK-C mediated gluconeogenesis.
Data show that PEPCK-M message and protein were detected in islets.
Title: Phosphoenolpyruvate cycling via mitochondrial phosphoenolpyruvate carboxykinase links anaplerosis and mitochondrial GTP with insulin secretion.

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Variation

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Alleles

Alleles of this type are documented at Mouse Genome Informatics (MGI)

Endonuclease-mediated (4) 1 citation
Targeted (1)

Interactions

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Products	Interactant	Other Gene	Complex	Source	Pubs	Description

General gene information

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Gene Ontology Provided by MGI

Function	Evidence Code	Pubs
enables GTP binding	IEA Inferred from Electronic Annotation more info
enables manganese ion binding	IBA Inferred from Biological aspect of Ancestor more info
enables manganese ion binding	ISO Inferred from Sequence Orthology more info
enables metal ion binding	IEA Inferred from Electronic Annotation more info
enables phosphoenolpyruvate carboxykinase (GTP) activity	IBA Inferred from Biological aspect of Ancestor more info
enables phosphoenolpyruvate carboxykinase (GTP) activity	IDA Inferred from Direct Assay more info	PubMed
enables phosphoenolpyruvate carboxykinase (GTP) activity	ISO Inferred from Sequence Orthology more info
enables phosphoenolpyruvate carboxykinase (GTP) activity	ISS Inferred from Sequence or Structural Similarity more info
enables phosphoenolpyruvate carboxykinase activity	ISO Inferred from Sequence Orthology more info

Process	Evidence Code	Pubs
involved_in NADH oxidation	ISO Inferred from Sequence Orthology more info
involved_in cellular response to dexamethasone stimulus	IBA Inferred from Biological aspect of Ancestor more info
involved_in cellular response to glucose stimulus	IBA Inferred from Biological aspect of Ancestor more info
involved_in cellular response to insulin stimulus	IBA Inferred from Biological aspect of Ancestor more info
involved_in gluconeogenesis	IBA Inferred from Biological aspect of Ancestor more info
involved_in gluconeogenesis	IEA Inferred from Electronic Annotation more info
involved_in gluconeogenesis	IGI Inferred from Genetic Interaction more info	PubMed
involved_in gluconeogenesis	ISO Inferred from Sequence Orthology more info	PubMed
involved_in glycerol biosynthetic process from pyruvate	IBA Inferred from Biological aspect of Ancestor more info
involved_in hepatocyte differentiation	IBA Inferred from Biological aspect of Ancestor more info
involved_in oxaloacetate metabolic process	IBA Inferred from Biological aspect of Ancestor more info
involved_in oxaloacetate metabolic process	ISO Inferred from Sequence Orthology more info
involved_in positive regulation of insulin secretion	ISO Inferred from Sequence Orthology more info
involved_in propionate catabolic process	IBA Inferred from Biological aspect of Ancestor more info
involved_in pyruvate metabolic process	ISO Inferred from Sequence Orthology more info
involved_in response to starvation	IBA Inferred from Biological aspect of Ancestor more info

Component	Evidence Code	Pubs
is_active_in mitochondrial matrix	IBA Inferred from Biological aspect of Ancestor more info
is_active_in mitochondrial matrix	IDA Inferred from Direct Assay more info	PubMed
located_in mitochondrion	HDA more info	PubMed
located_in mitochondrion	ISO Inferred from Sequence Orthology more info

General protein information

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Preferred Names: phosphoenolpyruvate carboxykinase [GTP], mitochondrial

Names: phosphoenolpyruvate carboxylase

NP_083270.2

EC 4.1.1.32

XP_030103944.1

EC 4.1.1.32

XP_030103945.1

EC 4.1.1.32

NCBI Reference Sequences (RefSeq)

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RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

mRNA and Protein(s)

NM_028994.3 → NP_083270.2 phosphoenolpyruvate carboxykinase [GTP], mitochondrial

Status: VALIDATED

Source sequence(s)

AK030501, BU757365

Consensus CDS

CCDS27114.2

UniProtKB/Swiss-Prot

Q8BH04, Q8BMM9, Q91Z10

UniProtKB/TrEMBL

Q3TDE6

Related

ENSMUSP00000038555.4, ENSMUST00000048781.4

Conserved Domains (1) summary

cd00819
Location:44 → 633

PEPCK_GTP; Phosphoenolpyruvate carboxykinase (PEPCK), a critical gluconeogenic enzyme, catalyzes the first committed step in the diversion of tricarboxylic acid cycle intermediates toward gluconeogenesis. It catalyzes the reversible decarboxylation and ...

RefSeqs of Annotated Genomes: GCF_000001635.27-RS_2024_02

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference GRCm39 C57BL/6J

Genomic

NC_000080.7 Reference GRCm39 C57BL/6J

Range

55777721..55787477

Download

GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

XM_030248084.2 → XP_030103944.1 phosphoenolpyruvate carboxykinase [GTP], mitochondrial isoform X1

UniProtKB/TrEMBL

Q3UQD8

Conserved Domains (1) summary

cl22860
Location:1 → 350

PEPCK_HprK; Phosphoenolpyruvate carboxykinase (PEPCK), a critical gluconeogenic enzyme, catalyzes the first committed step in the diversion of tricarboxylic acid cycle intermediates toward gluconeogenesis. It catalyzes the reversible decarboxylation and ...
XM_030248085.2 → XP_030103945.1 phosphoenolpyruvate carboxykinase [GTP], mitochondrial isoform X1

UniProtKB/TrEMBL

Q3UQD8

Conserved Domains (1) summary

cl22860
Location:1 → 350

PEPCK_HprK; Phosphoenolpyruvate carboxykinase (PEPCK), a critical gluconeogenic enzyme, catalyzes the first committed step in the diversion of tricarboxylic acid cycle intermediates toward gluconeogenesis. It catalyzes the reversible decarboxylation and ...